Grattan-Smith P J, Morris J G, Johnston H M, Yiannikas C, Malik R, Russell R, Ouvrier R A
Department of Paediatrics, Westmead Hospital, Sydney, Australia.
Brain. 1997 Nov;120 ( Pt 11):1975-87. doi: 10.1093/brain/120.11.1975.
The clinical and neurophysiological findings in six Australian children with generalized tick paralysis are described. Paralysis is usually caused by the mature female of the species Ixodes holocyclus. It most frequently occurs in the spring and summer months but can be seen at any time of year. Children aged 1-5 years are most commonly affected. The tick is usually found in the scalp, often behind the ear. The typical presentation is a prodrome followed by the development of an unsteady gait, and then ascending, symmetrical, flaccid paralysis. Early cranial nerve involvement is a feature, particularly the presence of both internal and external ophthalmoplegia. In contrast to the experience with North American ticks, worsening of paralysis in the 24-48 h following tick removal is common and the child must be carefully observed over this period. Death from respiratory failure was relatively common in the first half of the century and tick paralysis remains a potentially fatal condition. Respiratory support may be required for > 1 week but full recovery occurs. This is slow with several weeks passing before the child can walk unaided. Anti-toxin has a role in the treatment of seriously ill children but there is a high incidence of acute allergy and serum sickness. Neurophysiological studies reveal low-amplitude compound muscle action potentials with normal motor conduction velocities, normal sensory studies and normal response to repetitive stimulation. The biochemical structure of the toxin of I. holocyclus has not been fully characterized but there are many clinical, neurophysiological and experimental similarities to botulinum toxin.
本文描述了6例澳大利亚全身性蜱瘫痪患儿的临床和神经生理学表现。瘫痪通常由全环硬蜱的成熟雌蜱引起。最常发生在春夏季,但一年中的任何时候都可能出现。1至5岁的儿童最常受到影响。蜱通常见于头皮,常位于耳后。典型表现为先兆症状,随后出现步态不稳,然后是上行性、对称性、弛缓性瘫痪。早期累及脑神经是一个特点,尤其是动眼神经麻痹和外展神经麻痹同时存在。与北美蜱的情况不同,蜱去除后24 - 48小时内瘫痪加重很常见,在此期间必须对患儿进行密切观察。在本世纪上半叶,呼吸衰竭导致的死亡相对常见,蜱瘫痪仍然是一种潜在的致命疾病。可能需要呼吸支持超过1周,但患儿会完全康复。恢复过程缓慢,患儿需要数周时间才能独立行走。抗毒素在重症患儿的治疗中发挥作用,但急性过敏和血清病的发生率很高。神经生理学研究显示复合肌肉动作电位幅度降低,运动传导速度正常,感觉检查正常,对重复刺激反应正常。全环硬蜱毒素的生化结构尚未完全明确,但在临床、神经生理学和实验方面与肉毒杆菌毒素有许多相似之处。